BACKGROUND: So far there's no tumor maker applied in diagnosis and treatment of thymic epithelial tumors. This study is to assess the correlation between serum cytokine 19 fragment (Cyfra 21-1) and clinicopathological features and prognosis of thymic epithelial tumors (TETs). METHODS: The clinical data of 159 patients with TETs in Shanghai Chest Hospital was retrospectively analysed. Patients were divided into groups according to different tumor stages and histotypes. Serum Cyfra 21-1 was thus compared. In addition, the possible relationship between perioperative serum Cyfra 21-1 level and the recurrent status was carrid out. RESULTS: Preoperative Cyfra 21-1 serum concentrations in patiants with advanced stage (T4) and thymic carcinomas were significantly higher than that in others (P<0.001, P<0.001, respectively). When the preoperative serum level exceeds the out-off of 1.66 ng/mL, it possibly indicates the recurrence during follow up. Furthermore, the sensitivity, specificity, and positive as well as negative predictive value (PPV and NPV) of postoperative Cyfra 21-1 to predict tumor recurrence were evaluated. At a cut-off of Cyfra 21-1 of 2.66 ng/mL, the sensitivity was 0.7, the specificity was 0.925, the PPV was 0.5 and the NPV was 0.966. CONCLUSIONS The elevated level of preoperative serum Cyfra 21-1 indicates an advanced stage of tumor or a more malignant histotype (thymic carcinoma). It also probably suggests a higher risk of tumor recurrence. During the oncological follow up, in addition to regular imaging examinations, the blood test of serum Cyfra 21-1 is also suggested to improve the diagnosis of tumor recurrence in order to improve the prognosis.
Biomarkers, Tumor ; blood ; Female ; Follow-Up Studies ; Humans ; Keratin-19 ; blood ; chemistry ; Male ; Middle Aged ; Neoplasms, Glandular and Epithelial ; blood ; diagnosis ; pathology ; Peptide Fragments ; blood ; Prognosis ; ROC Curve ; Retrospective Studies ; Thymus Neoplasms ; blood ; diagnosis ; pathology

Humans ; Pathology, Molecular ; Prognosis ; Thymoma ; diagnosis ; pathology ; Thymus Neoplasms ; diagnosis ; pathology

OBJECTIVETo review the presentation, diagnosis, staging and treatment of thymoma.

DATA SOURCESData were obtained from papers on thymoma published in English within the last 30 years. No formal systematic review was conducted, but an effort was made to be comprehensive.

STUDY SELECTIONStudies were selected if they contained data relevant to the topic addressed in the particular section. In particular, standards adopted by the International Thymic Malignancies Interest Group through a formal process of achieving worldwide consensus are featured. Because of the limited length of this article, we have frequently referenced recent reviews that contain a comprehensive amalgamation of literature rather than the actual source papers.

RESULTSThymomas are rare malignant tumors. They account for about half (47%) of anterior mediastinal tumors. About one third of these are associated with myasthenia gravis. Computed tomography with intravenous contrast is the standard diagnostic modality. Thymomas appear as round or oval masses in early stages but irregular shapes with calcifications occurring in later stages. They can invade surrounding structures including mediastinal fat, pleura, major blood vessels and nerves. Fine needle aspiration, core needle biopsy or open biopsy is used to obtain tissue diagnosis. Masaoka-Koga classification is currently used to stage thymomas. All thymomas should be considered for resection due to their malignant potential. A complete resection is a major prognostic factor and every effort should be made to achieve this even if this means resection and reconstruction of a major thoracic structure. Median sternotomy is the standard approach for thymoma resection. A number of minimally invasive techniques are used in selective centers. While stage I and II tumors undergo primary surgery, preoperative chemotherapy appears to increase the chances of complete resection for stage III and IVa tumors. Postoperative radiation could be considered for patients with residual disease. Excellent 5 and 10-year survival rates are noted for completely resected early stage thymomas.

CONCLUSIONSThymic malignancies are rare tumors. Standards have recently been achieved to allow better communication and promote collaborative research. Surgical resection is the mainstay of treatment, but a multimodality approach is useful for many patients.

Humans ; Incidence ; Neoplasm Recurrence, Local ; therapy ; Neoplasm Staging ; Thymoma ; diagnosis ; pathology ; therapy ; Thymus Neoplasms ; diagnosis ; pathology ; therapy ; Tomography, X-Ray Computed

Adenocarcinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Carcinoembryonic Antigen ; metabolism ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Keratin-20 ; metabolism ; Male ; Microfilament Proteins ; metabolism ; Middle Aged ; Phosphopyruvate Hydratase ; metabolism ; Thymus Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT).

METHODSThe clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features, treatment and prognosis were discussed and literatures reviewed.

RESULTSOne male and two female patients presented with asymptomatic mediastinal masses with a history of Sjögren syndrome. They were aged 36, 35 and 41 years respectively, and only one patient had B symptoms. Grossly, all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface. Histopathologically, the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation. Immunohistochemically, the tumor cells were positive for CD20, CD79a, bcl-2 and negative for CD3, CD5, cyclin D1, CD43, CD10, bcl-6, and CD23. The plasma cells showed kappa light chain restriction. Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR. All patients were at early stage and received routine chemotherapy with or without radiotherapy after surgical removal. All patients achieved complete remission with 24, 18 and 3 months follow-up, respectively.

CONCLUSIONSPrimary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation, other types of lymphoma and mediastinal thymoma.

Adult ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; Humans ; Immunoglobulin Heavy Chains ; genetics ; Keratin-19 ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Male ; Prednisone ; therapeutic use ; Pseudolymphoma ; pathology ; Thymus Hyperplasia ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

Adult ; CD5 Antigens ; metabolism ; Carcinoma, Papillary ; metabolism ; pathology ; Carcinoma, Squamous Cell ; metabolism ; pathology ; surgery ; Choristoma ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Hamartoma ; metabolism ; pathology ; Humans ; Proto-Oncogene Proteins c-kit ; metabolism ; Thymoma ; metabolism ; pathology ; Thymus Gland ; pathology ; Thymus Neoplasms ; metabolism ; pathology ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods

Adult ; Antigens, CD20 ; metabolism ; Carcinoma ; metabolism ; pathology ; DNA Nucleotidylexotransferase ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Keratins ; metabolism ; Lymphoma ; metabolism ; pathology ; Male ; Middle Aged ; Necrosis ; Seminoma ; metabolism ; pathology ; Thymoma ; metabolism ; pathology ; surgery ; Thymus Neoplasms ; metabolism ; pathology ; surgery ; Tuberculosis ; pathology

Child ; Female ; Head and Neck Neoplasms ; diagnosis ; pathology ; surgery ; Humans ; Parathyroid Neoplasms ; diagnosis ; pathology ; surgery ; Thymus Gland ; pathology ; surgery ; Thymus Neoplasms ; diagnosis ; pathology ; surgery

Carcinoma ; drug therapy ; genetics ; metabolism ; pathology ; radiotherapy ; Desmoplastic Small Round Cell Tumor ; metabolism ; pathology ; Diagnosis, Differential ; Gene Rearrangement ; Head and Neck Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Humans ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Lymphatic Metastasis ; Male ; Mediastinal Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Melanoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Nuclear Proteins ; genetics ; metabolism ; Oncogene Proteins ; genetics ; metabolism ; Oncogene Proteins, Fusion ; genetics ; metabolism ; Rhabdomyosarcoma ; metabolism ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy

Aged ; CD5 Antigens ; metabolism ; Carcinoma, Basal Cell ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Lung Neoplasms ; pathology ; Male ; Mediastinal Cyst ; pathology ; Middle Aged ; Proto-Oncogene Proteins c-kit ; metabolism ; Thymoma ; pathology ; Thymus Neoplasms ; metabolism ; pathology ; surgery